Vulvar skin disease in the pediatric population.

PURPOSE OF REVIEW: Vulvar skin disease is an underrecognized pediatric condition encompassing a wide range of conditions, from isolated vulvar disease to vulvar manifestations of systemic illnesses. This review highlights the most current research discussing clinical features, risk factors, and treatments. RECENT FINDINGS: Recent studies confirm that labial adhesions resolve more quickly with estrogen treatment. Topical corticosteroids remain first-line for treatment of vulvar lichen sclerosus, and some procedural interventions are showing promising results. Latest evidence shows efficacy of biologic agents in hidradenitis suppurativa and vulvar Crohn's. Vaginal voiding remains an underrecognized cause of irritant vulvovaginitis. Lately vulvar aphthae have been associated with coronavirus disease 2019. Distinguishing between infantile perianal pyramidal protrusion, molluscum, and condyloma can be aided by differences in morphologic features. SUMMARY: Vulvar dermatoses have a high impact on children's health and wellbeing. Clinician familiarity with recognition and latest advancements in vulvar dermatoses can aid in prompt diagnosis, management, and appropriate referrals. Vulvar biopsy and vaginal cultures should be used prudently. Treatments include topicals, behavioral modification, systemic medications, and procedures.

Lichen Sclerosus: A Survey of Diagnosis and Management Among Pediatric Dermatologists and Gynecologists.

BACKGROUND/OBJECTIVES: Lichen sclerosus (LS) is a chronic condition that warrants close follow-up due to the risk of scarring. The optimal long-term management of pediatric vulvar and perianal lichen sclerosus (PVPLS) is unknown. This study aimed to identify diagnostic, treatment, and maintenance regimens among pediatric dermatologists and pediatric/adolescent gynecologists, as well as assess provider confidence and desire for guidance on long-term PVPLS management. METHODS: A cross-sectional 35-question survey was administered through the Pediatric Dermatology Research Alliance (PeDRA) and the North American Society for Pediatric and Adolescent Gynecology (NASPAG) between 7/13/2021 and 8/30/2021 to ascertain PVPLS diagnostic and management regimens. RESULTS: Most responders were attending-level pediatric/adolescent gynecologists (46%) and pediatric dermatologists (41%). Although 85% of participants felt completely or very confident in diagnosing PVPLS, the majority (86%) desired further management guidelines. While the initial treatment was similar among providers, maintenance regimens and follow-up varied considerably, with only 42% recommending lifelong monitoring despite potential persistence into adulthood. CONCLUSIONS: While initial treatment was similar among practitioners, there was variation by specialty in subsequent management and a lack of uniformity in long-term follow-up. Additional studies are needed to clarify the optimal management of PVPLS and to provide evidence-based guidelines regarding long-term follow-up.  J Drugs Dermatol. 2024;23(6):450-455.     doi:10.36849/JDD.8084.

Atopic Dermatitis: Managing the Itch.

Atopic dermatitis has a substantial impact on sleep, appearance, psychological well-being, and other qualities of life. The visual appearance of lichenification, cheilitis, hyperpigmentation, ichthyosis, and erythema can be socially stigmatizing, and treatment of these symptoms is challenging. In managing pruritus in patients, practitioners should assess and document pruritus through questionnaires at each routine visit. Initially, practitioners should advise patients to employ nonpharmaceutical treatments such as emollients with wet wraps, elimination of triggers, changing scratching habits, and psychological interventions. If these methods of treatment are not successful or if the disease presentation is severe, pharmacological therapies should be employed. This chapter describes the therapeutic ladder for pruritus in atopic dermatitis and discusses each treatment modality in further detail for practitioners to advise their patients.First-line topical pharmaceutical agents include topical glucocorticoids and topical calcineurin inhibitors. Second-line topical agents include coal tar, menthol, capsaicin, or doxepin. After the use of topical agents has been exhausted, primary systemic agents can be applied. These include sedating antihistamines, nonsedating antihistamines, oral glucocorticoids, or cyclosporine A. Finally, neuromodulating or immunomodulating agents can be attempted, including SSRI/SNRIs, TCAs, immunosuppressants, neural modulators, and opioid receptor modulators. Outside of pharmacological treatments, phototherapy has been shown to provide a dramatic improvement of pruritus in atopic dermatitis and can be used at any stage of treatment including as a first-line agent.

Characteristics and outcomes for participants with congenital ichthyosis who responded to treatment with the topical isotretinoin formulation TMB-001: results from the Phase IIb CONTROL study.

BACKGROUND: Emollients and keratolytics are frequently used to manage symptoms of congenital ichthyosis (CI). Systemic retinoid treatment is complicated by teratogenicity and dose-limiting adverse effects. OBJECTIVES: This analysis from the randomized Phase IIb CONTROL study investigated the characteristics of participants who responded to treatment with TMB-001, a novel topical isotretinoin ointment formulation. METHODS: Participants ≥ 9 years of age with genetically confirmed CI and ≥ 2 (out of 4) Visual Index for Ichthyosis Severity (VIIS) assessment areas with ≥ 3 scaling score were randomized 1 : 1 : 1 to TMB-001 0.05%, TMB-001 0.1% or vehicle, twice daily for 12 weeks. Efficacy endpoints included the proportion of participants with ≥ 50% reduction in VIIS-scaling (VIIS-50) compared with baseline and ≥ 2-grade reduction in Investigator's Global Assessment (IGA)-scaling score compared with baseline. Changes in body surface area (BSA) involvement, Dermatology Life Quality Index (DLQI) scores and Itch-Numeric Rating Scale (I-NRS) scores were assessed. RESULTS: Among the 33 participants (11 randomized to TMB-001 0.05%, 10 to TMB-001 0.1% and 12 to vehicle), median age was 29 years (range 9-80), and most were male (64%) and White (79%). Baseline demographics were generally similar among participants who did or did not achieve TMB-001 treatment success. Participants who had lower mean BSA involvement and higher DLQI and I-NRS scores at baseline were more likely to achieve VIIS-50. Similarly, higher baseline DLQI and I-NRS scores were associated with IGA response; BSA involvement was similar for IGA responders vs. nonresponders. CONCLUSIONS: Higher DLQI and I-NRS scores at baseline were associated with participants achieving treatment success by VIIS-50 and IGA response. Lower BSA involvement was associated with VIIS-50 success.

Phase IIb randomized CONTROL study demonstrates a novel topical isotretinoin formulation, TMB-001, is safe and effective in participants with either recessive X-linked or autosomal recessive lamellar congenital ichthyosis.

BACKGROUND: In two severe congenital ichthyosis subtypes, autosomal recessive lamellar ichthyosis (ARCI-LI) and X-linked recessive ichthyosis (XLRI), cutaneous manifestations include widespread scaling. Approved topical treatment options are limited to emollients and keratolytics. AIM: This analysis from the randomized phase IIb CONTROL study assessed whether the efficacy and safety of TMB-001, a novel topical isotretinoin ointment formulation, differed between ARCI-LI and XLRI subtypes. METHODS: Participants ≥ 9 years with genetically confirmed XLRI or ARCI-LI and ≥ 2 (of 4) Visual Index for Ichthyosis Severity (VIIS) assessment areas with ≥ 3 scaling score were randomized 1 : 1 : 1 to TMB-001 0.05%/TMB-001 0.1%/vehicle, twice daily for 12 weeks. The proportion of participants with ≥ 50% reduction vs. baseline in VIIS scaling (VIIS 50; primary endpoint) and ≥ 2-grade reduction in Investigator's Global Assessment (IGA)-scaling score vs. baseline (key secondary endpoint) were evaluated. Adverse events (AEs) were monitored. RESULTS: Among enrolled participants (TMB-001 0.05%, n = 11; 0.1%, n = 10; and vehicle, n = 12), 52% had ARCI-LI and 48% XLRI subtypes. Mean age was 33.6 and 35.4 years for participants with ARCI-LI and XLRI, respectively. Overall, 33%, 50% and 17% of participants with ARCI-LI and 100%, 33% and 75% of participants with XLRI achieved VIIS 50 in the TMB-001 0.05%, TMB-001 0.1% and vehicle groups, respectively (nominal P = 0.24 for 0.05% vs. vehicle, intent-to-treat population). Improvement of ≥ 2-grade IGA score was observed in 33%, 50% and 0% of participants with ARCI-LI and 83%, 33% and 25% of participants with XLRI in the TMB-001 0.05%, TMB-001 0.1% and vehicle groups, respectively (nominal P = 0.03 for 0.05% vs. vehicle, intention-to-treat population). Most AEs were application-site reactions. CONCLUSION: Regardless of congenital ichthyosis subtype, TMB-001 demonstrated greater proportions of participants achieving VIIS 50 and ≥ 2-grade IGA improvement vs. vehicle.

Prevalence of delayed puberty and low bone density in patients with epidermolysis bullosa: Insight from a large single center's experience.

BACKGROUND: Epidermolysis bullosa (EB) is a group of rare genetic skin conditions that result in skin fragility. EB can be quite severe with chronic inflammation and malnutrition impairing growth and pubertal development. These factors have potential consequences for skeletal health. We aimed to determine the prevalence of delayed puberty and low bone mineral density (BMD) for age in children and young adults with EB. METHODS: Electronic medical records (EMR) of patients with confirmed EB <30 years of age at time of initial encounter at Cincinnati Children's Hospital Medical Center between January 1, 2010 and September 30, 2020 were reviewed. Natural language processing software was used to categorize pubertal status of patients with EB as early, normal or delayed. BMD was measured by dual energy x-ray absorptiometry and categorized as low if height adjusted Z-score was <-2.0 using age, sex and race specific reference ranges. RESULTS: 29% of individuals with EB had low BMD with most cases occurring prior to 10 years of age. Of patients who reached adolescence, 23% failed to develop any signs of puberty in the normal range (before age 13 in females or 14 in males) and BMD Z-scores further declined in these individuals. CONCLUSION: Delayed puberty is an under-recognized comorbidity of individuals with EB, especially in those with recessive dystrophic EB, and can have a significant impact on BMD.

Epidermolytic ichthyosis complicated by staphylococcal scalded skin syndrome in the newborn.

Epidermolytic ichthyosis is characterized by erythema and blistering at birth. We present a neonate with epidermolytic ichthyosis who had a subtle change in clinical findings while hospitalized, including increased fussiness, erythema, and a change in her skin odor, which represented superimposed staphylococcal scalded skin syndrome. This case highlights the unique challenge of recognizing cutaneous infections in neonates with blistering skin disorders and emphasizes the importance of having a high suspicion for superinfection in this population.

Most common pediatric skin conditions managed in outpatient dermatology clinics in the United States stratified by race and ethnicity.

A better understanding of what skin conditions are most commonly diagnosed in different pediatric racial and ethnic groups in outpatient dermatology clinics could help guide the development of pediatric dermatology educational initiatives for primary care providers and general dermatologists who have limited access to pediatric dermatologists. Using a nationally representative dataset, we evaluated the most common diagnoses in patients younger than 15 years of age (children) and 15-24 years of age (youth) who present to outpatient dermatology clinics, stratified by race and ethnicity. While acne and dermatitis were among the top ten most common diagnoses in all racial and ethnic groups studied, Black children were also commonly diagnosed with dermatophytosis and impetigo, and Black and Hispanic children were often diagnosed with seborrheic dermatitis; pigmentary disorders were among the top three most common diagnoses in Black, Asian, and Hispanic youth. Training more physicians how to evaluate and treat common skin conditions in children and youth of diverse racial and ethnic backgrounds may improve access to care for skin disease in the United States.

Vulvar diseases: Conditions in adults and children.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Vulvar diseases: Approach to the patient.

Patients with vulvar dermatoses often delay seeking medical treatment because of anxiety and embarrassment. Moreover, women frequently self-treat with various home remedies and see multiple clinicians before presenting to a dermatologist. Despite serving as the primary providers for patients with vulvovaginal symptoms, gynecologists typically receive limited training in the causes and management of these conditions. Dermatologists are experts in the evaluation and management of cutaneous disease and should be the caretakers of all skin, including the genitalia. Vulvar disorders are underrecognized by dermatologists for numerous reasons: inadequate training, lack of comfort with both interview and examination techniques, and unfamiliarity with normal anatomic variations. The first article in this continuing medical education series on vulvar dermatoses reviews the fundamentals, approach, and techniques that can be used to ensure a successful visit for both patient and provider.

Enteral iron absorption in patients with recessive dystrophic epidermolysis bullosa.

BACKGROUND/OBJECTIVES: To determine whether iron was being enterally absorbed in anemic patients with recessive dystrophic epidermolysis bullosa (RDEB). METHODS: Anemic patients with RDEB who were refractory or had poor adherence to oral or gastrostomy-given iron underwent enteral iron absorption challenges. Subjects were given 2 mg/kg of elemental iron. Successful iron absorption was defined as a two- to threefold increase of serum iron or a rise to above 100 µg/dL. RESULTS: Nine of 12 iron challenges did not show increased iron absorption. Only three of the ten subjects demonstrated elevated iron absorption. All patients had elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), low serum albumin, and hemoglobin levels. Eight challenges were in patients with elevated soluble transferrin receptor (STFR)/log ferritin levels, indicating iron deficiency. The three challenges with elevated iron absorption also had elevated STFR/log ferritin as well as elevated ESR and CRP, but these inflammatory markers were, in general, less elevated than those in non-absorbers. CONCLUSIONS: Enteral iron is routinely prescribed for anemic patients with RDEB assuming a component of iron deficiency. Adherence to enteral iron tends to be unreliable due to unpalatable taste and gastrointestinal complaints. Enteral iron absorption tests are relatively noninvasive and appear to be well tolerated. Poor gastrointestinal iron absorption may be an important factor in failure to improve anemia in RDEB enterally. It may be prudent to test patients with RDEB who are anemic and not responding well to conventional iron supplements with iron absorption tests and to consider replacement with intravenous iron in iron-deficient patients.

Inner thigh friction as a cause of acne mechanica.

Acne mechanica is defined as being any acneiform eruption in areas of friction, pressure, stretching, rubbing, pinching, or occlusion of the skin in any individual, regardless of preexisting acne. Various causes have been reported, including prolonged back rest against a chair or bed, occlusive clothing, pressure from a prosthetic limb, and others. This is the first reported case of bilateral open comedones caused by inner thigh friction.

A case of verrucous perforating collagenoma in a toddler.

Verrucous perforating collagenoma is an extremely rare variant of acquired perforating dermatosis that has been seldom described in literature. We present the case of an 18-month-old boy who presented with an erythematous plaque with a central keratotic plug on the leg. Histopathology revealed transepidermal elimination of collagen, consistent with a diagnosis of verrucous perforating collagenoma.

Idiopathic toxic epidermal necrolysis in an adolescent.

A 10-year-old girl, suspected 2 days prior to have streptococcal pharyngitis, presented with diffuse erythema, tense bullae, Nikolsky-positive desquamation, as well as ulcerations of her oral and genital mucosa. She denied recent travel, sick contacts, or preceding and concurrent use of medications, including over-the-counter and herbal supplements. A comprehensive viral polymerase chain reaction (PCR) panel, Mycoplasma pneumoniae PCR and IgM, streptococcal molecular antigen test, urine culture, blood culture, and rheumatologic serologies were negative. Based on the patient's clinical presentation and biopsy results, she was diagnosed with idiopathic toxic epidermal necrolysis.

Guidelines for pediatric anogenital examination: Insights from our vulvar dermatology clinic.

To our knowledge, there are no guidelines in the dermatology literature for performing anogenital examinations in prepubescent children. Based on experience in our joint pediatric dermatology-gynecology vulvar clinic, we aim to provide a framework for conducting genital examinations in children, focusing on the vulvar examination. Our goal is to enhance confidence in the pediatric dermatologist's ability to perform thorough examinations by providing general principles as well as concrete "do's" and "don'ts" that will create a maximally comfortable and productive experience. These steps will help create a positive experience for the patient and family, encouraging further follow-up and enhancing the overall well-being of the child.

Erythema ab igne in patients with sickle cell disease.

Erythema ab igne (EAI) is an asymptomatic dermatosis caused by prolonged exposure to localized heat. Affected areas have net-like hyperpigmentation that may resemble more serious conditions such as livedo racemosa or vasculitis. We report three cases of EAI in pediatric sickle cell disease (SCD) patients who were initially suspected of having a more severe, life-threatening disorder before Dermatology was consulted. Clinicians caring for pediatric SCD patients who regularly use heating pads/devices for pain relief should consider EAI in the differential diagnosis of large areas of net-like hyperpigmentation. This paper aims to increase recognition of EAI and patient education on safe practices while using heating pads.

Atopic Dermatitis: Managing the Itch.

Atopic dermatitis has a substantial impact on sleep, appearance, psychological well-being, and other qualities of life. The visual appearance of lichenification, cheilitis, hyperpigmentation, ichthyosis, and erythema can be socially stigmatizing, and treatment of these symptoms is challenging. In managing pruritus in patients, practitioners should assess and document pruritus through questionnaires at each routine visit. Initially, practitioners should advise patients to employ non-pharmaceutical treatments such as emollients with wet wraps, elimination of triggers, changing scratching habits, and psychological interventions. If these methods of treatment are not successful or if the disease presentation is severe, pharmacological therapies should be employed. This chapter describes the therapeutic ladder for pruritus in atopic dermatitis and discusses each treatment modality in further detail for practitioners to advise their patients.First-line topical pharmaceutical agents include topical glucocorticoids and topical calcineurin inhibitors. Second-line topical agents include coal tar, menthol, capsaicin, or doxepin. After the use of topical agents has been exhausted, primary systemic agents can be applied. These include sedating antihistamines, non-sedating antihistamines, oral glucocorticoids, or cyclosporine A. Finally, neuromodulating or immunomodulating agents can be attempted, including SSRI/SNRIs, TCAs, immunosuppressants, neural modulators, and opioid receptor modulators. Outside of pharmacological treatments, phototherapy has been shown to provide a dramatic improvement of pruritus in atopic dermatitis and can be used at any stage of treatment including as a first-line agent.

The Fanny Pack: No Ifs, Ands, or Buts.

Although the white coat is central to the practice of adult medicine, pediatricians often shed it to avoid creating negative associations and provoking fear in children. In our pediatric dermatology practice, the fanny pack (FP) has replaced many of the functional elements of the white coat. The FP is a kid-friendly way to readily carry key medical supplies from one patient encounter to the next. It is more hygienic, light weight, and affordable than traditional doctor's bags and white coats. In this article we outline the benefits of the FP, which include storage, ergonomics, efficiency, cleanliness, kid-friendliness, and cost-effectiveness.

Eruptive milia during isotretinoin therapy.

Isotretinoin (13-cis-retinoic acid) is a synthetic vitamin A derivative that is effective in the treatment of recalcitrant, nodulocystic acne. To our knowledge, there are no reports in the medical literature of milia as a side effect of isotretinoin. We report a case of eruptive facial milia in the setting of isotretinoin treatment for acne.